Miyasaka H, Nohara C, Ohtani H, Suda K, Mori H, Nakajima Y, Mizuno Y
Department of Neurology, Juntendo University School of Medicine, Tokyo.
No To Shinkei. 1994 Nov;46(11):1101-11.
We report a 65-year-old woman with progressive multiple cranial neuropathy. She had been suffered from bronchial asthma since 1979 for which prednisolone had been prescribed. She noted an onset of pain around her nose in October, 1989, which extended into the periorbital regions bilaterally. In February, 1990, she was treated with stellate ganglion block and trigeminal nerve block; these treatments partially alleviated her pain. In May of 1991, she noted a difficulty in swallowing solid foods. In November of the same year, she developed right facial paresis; two weeks later, she noted numbness in her left face, and was hospitalized to our service on December 16, 1991. On admission, she was afebrile and general physical examination was unremarkable except for piping rales in her both lung fields. On neurologic examination, she was alert and oriented to all spheres; higher cerebral functions were intact. In the cranial nerves, her olfactory sense was lost bilaterally; her vision was markedly diminished bilaterally only to recognize hand movements; the optic fundi appeared normal; the pupils were isocoric and reacted to light promptly. The extraocular muscles were moderately weak to most of the directions more on the left; no nystagmus was present. Facial sensation was diminished bilaterally; the jaw deviated to right; right facial paresis of peripheral type was present; her hearing was diminished bilaterally more on the right. The movement of the soft palate was diminished on the right side; dysphagia was present; her voice was horse; the gag reflex was diminished. The sternocleidomastoid muscle was weak bilaterally; the tongue appeared normal. Examination of gait was differed because of headache, however, no apparent motor weakness was present. No ataxia or involuntary movement was noted. Deep reflexes were normally elicited and symmetric. Plantar response was flexor. Sensation in the extremities was intact. Kernig's sign was positive at 70 degree leg extension; eyeball tenderness was also present bilaterally, however, no nuchal stiffness was noted. Following abnormalities were present in the laboratory examination: WBC 11,400/microliters, ESR 50 mm/hr, CRP 6.1 mg/dl. The lumbar CSF was under a normal pressure containing 29 WBC/microliters (neutrophils 7, lymphocytes 20, others 2), 67 mg/dl of protein, and 53 mg/dl of sugar; cultures for acid-fast bacilli as well as for other bacteria were negative; no malignant cells were found. A cranial CT scan revealed an isodensity mass in the orbit and ill-defined low density areas in the white matters of the frontal lobes.(ABSTRACT TRUNCATED AT 400 WORDS)
我们报告一名65岁患有进行性多发性颅神经病变的女性。她自1979年起患有支气管哮喘,一直服用泼尼松龙。1989年10月,她注意到鼻子周围开始疼痛,并双侧蔓延至眶周区域。1990年2月,她接受了星状神经节阻滞和三叉神经阻滞治疗;这些治疗部分缓解了她的疼痛。1991年5月,她注意到吞咽固体食物困难。同年11月,她出现右侧面部轻瘫;两周后,她注意到左侧面部麻木,并于1991年12月16日入住我院。入院时,她无发热,全身体格检查除双肺野有管状啰音外无异常。神经系统检查时,她意识清醒,对所有方面定向正常;高级脑功能完好。在颅神经方面,她双侧嗅觉丧失;双侧视力明显减退,仅能识别手部动作;眼底外观正常;瞳孔等大,对光反应迅速。双眼外肌在大多数方向上中度无力,左侧更明显;无眼球震颤。双侧面部感觉减退;下颌偏向右侧;存在右侧周围性面神经麻痹;双侧听力减退,右侧更明显。右侧软腭运动减弱;存在吞咽困难;声音嘶哑;咽反射减弱。双侧胸锁乳突肌无力;舌头外观正常。由于头痛,步态检查受限,但未发现明显运动无力。未发现共济失调或不自主运动。深反射正常引出且对称。跖反射为屈性。四肢感觉正常。直腿抬高70度时克氏征阳性;双侧眼球也有压痛,但未发现颈项强直。实验室检查有以下异常:白细胞11,400/微升,血沉50毫米/小时,C反应蛋白6.1毫克/分升。腰穿脑脊液压力正常,含白细胞29/微升(中性粒细胞7个,淋巴细胞20个,其他2个),蛋白67毫克/分升,糖53毫克/分升;抗酸杆菌及其他细菌培养均为阴性;未发现恶性细胞。头颅CT扫描显示眼眶内有等密度肿块,额叶白质有边界不清的低密度区。(摘要截取自400字)